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PubMed1978

The response of young chronically protein-deficient mice to multiple antigenic challenge.

Responses to two or more antigens given together or separated by an interval of up to four days were determined in young mice maintained from weaning on a 4% albumin diet, and compared with those of normally-fed controls. Simultaneous challenge with several antigens did not affect antibody production in mice of either dietary group ("Simultaneous Competition") and the responses of normal and protein-deficient mice to sheep or rat erythrocytes were similarly produced by the prior injection of horse or sheep cells ("Sequential Competition"). However, the low protein diet broadened the conditions under which primary and secondary injections of diphtheria toxoid impaired primary responses to tetanus toxoid. These findings are discussed in relation to mechanisms previously proposed to explain antigenic competition, with a view to explaining the effects of protein-deficiency on antibody production.

Adsorption↗
PubMed1989

A Dutch family with hereditary protein S deficiency.

Protein S, a vitamin K dependent factor, acts as a cofactor for activated protein C in preventing coagulation and stimulating fibrinolysis. Hereditary protein S deficiency has been reported to be an autosomal dominant disorder, associated with an increased risk for developing thrombosis in heterozygotes. Here we present a large Dutch family with familial thrombophilia based on hereditary protein S deficiency. Besides the proband, 27 individuals were tested. Of these, four had had complaints of thromboembolic events. Three of them had protein S levels below the limits of normal, and were considered to be heterozygous for protein S deficiency. Ten others who were also found to be heterozygotes had had no manifestations. Seven of them were under 15 years of age at the time of the investigation. It is uncommon for heterozygotes with protein S deficiency to develop thrombosis before that age, although there have been a few reports. Following these observations, some remarks are made on how to make the laboratory diagnosis of the deficiency, on when to perform family choice of analysis, and on the consequences for therapy.

Adolescent↗
PubMed2000

Rolipram does not normalize very long-chain fatty acid levels in adrenoleukodystrophy protein-deficient fibroblasts and mice.

In its severe form, X-linked adrenoleukodystrophy (X-ALD) is a lethal neurodegenerative disorder with inflammatory demyelination, in which defective peroxisomal beta-oxidation causes accumulation of very long-chain fatty acids (VLCFA) in tissues and plasma, in particular in the nervous system and adrenal glands. Recently, several drugs have been reported to reduce VLCFA in cultured human fibroblasts of X-ALD patients, and therefore to be potential candidates for novel therapeutic treatments in X-ALD. Among the most promising of these substances is the antidepressant rolipram, because of favourable adverse event profile in clinical studies and its additionally reported anti-inflammatory action. To further elucidate the effects of rolipram on peroxisomal beta-oxidation and VLCFA accumulation, we administered rolipram orally in the diet to ALD protein-deficient mice and ALD protein-deficient cultured human and mouse fibroblasts and assayed the accumulation of VLCFA. In contrast to the previously reported reduction of VLCFA, our data did not demonstrate a decrease in VLCFA content either in vivo or in vitro. NMR spectroscopic analysis verified the structural integrity and purity of the rolipram used here, thus excluding inauthenticity as a reason for the discrepancy. We therefore suggest that rolipram should be excluded from the current list of potential therapeutic agents for X-ALD.

ATP Binding Cassette Transporter, Subfamily D, Mem↗
PubMed1975

The modification of the renal carcinogenicity of dimethylnitrosamine by actinomycin D and a protein deficient diet.

The effect of a single treatment with 30 mg dimethylnitrosamine (DMN) and 6 mug actinomycin D (ACT), given at different time intervals (ACT application to DMN, 2 h before, simultaneously, 5, 9 or 48 h later), was tested in female Sprague-Dawley rats in relation to renal carcinogenesis; additionally, the animals were fed either a normal or a protein deficient diet. The ACT treatment did not significantly modify either the kidney tumour incidence or the survival time in the different groups fed a normal diet. Nevertheless, there are indications that additional ACT application may shorten the latency period for DMN induced renal neoplasms or, when administered 5 h later than DMN, a slightly decreased and delayed tumour induction can be assumed. In groups fed a protein deficient diet, a significantly higher percentage of kidney tumour bearing animals as well as a shortened latency period were found when compared with the DMN group on normal diet, but these differences were independent of the additional ACT treatment 9 h later than DMN and were due to the protein deprivation. Morphologically, the tumours were of epithelial and mesenchymal type with a clear preponderance of the former type. Biochemical and morphological aspects are discussed.

Adenoma↗
PubMedDate not supplied

Red cell membrane protein deficiencies in Mexican patients with hereditary spherocytosis.

Twenty-seven families and four individual patients with hereditary spherocytosis (HS) from the northwestern region of Mexico were studied. An autosomal dominant inheritance pattern was identified in 59% of 22 families. Densitometric analysis of erythrocyte membrane proteins revealed individual protein deficiencies in 39% of the patients studied, in whom the principal altered proteins were the alpha spectrins (13%), band 3 protein (10%), ankyrin (6%), 4.2 protein (6%), and the beta spectrins (3%). A predominant deficiency of spectrins has also been observed in other Latin American and Mediterranean countries. However, it is well known that deficiencies in these proteins are heterogeneous across different ethnic groups. A combined protein deficiency was observed in 52% of patients, most frequently involving the spectrins, band 3 protein, 4.2 protein, and 4.1 protein. In three subjects, no abnormalities were detected (10%). We conclude that, despite the observed heterogeneity, the principal affected proteins are essentially similar to those observed in other ethnic groups.

Adolescent↗
PubMed1977

[Effect of orally administered pancreatic extract on Vibrio cholerae infection during protein deficiency].

Two groups of 5 and 6 Vervet monkeys respectively were fed a protein-free diet. Both groups of protein depleted Vervets developed a minor decrease of serum and intestinal immunoglobulins, as well as marked impairment of exocrine pancreatic function. Thereafter the groups were challenged with Vibrio cholerae, while one group received purified pancreatic extract by oral administration. The group without pancreatic extract developed severe and significantly longer lasting diarrhea than the group with pancreatic extract. Vibrios were excreted for much longer in the former group. It is concluded that exocrine pancreatic function is involved in the local defence mechanism against cholera during protein deficiency.

Animals↗
PubMed1986

Effect of protein deficiency and Tween 60 on the pharmacokinetics of butylated hydroxyanisole and metabolites in male Sprague-Dawley rats.

Radioactive 3H-butylated hydroxyanisole (BHA) at dosages of 250 or 2,500 mg/kg body weight was administered to rats either orally (po) or intraperitonealy (ip) with or without 500 mg Tween 60/kg. The pharmacokinetics of 3H-BHA were evaluated in both control and protein-deficient animals. Blood or urine and feces were collected and analyzed for 3H-BHA equivalents. Using the Autoan/Nonlin69 computer program, most pharmacokinetic profiles of BHA (250 mg/kg) were fit to one-compartment open models with first-order absorption. The exception was the model for ip administration, which resulted in a two-compartment open model with first-order absorption. Increasing the dose of BHA to 2,500 mg/kg altered pharmacokinetics of BHA; no decline in blood levels was observed 48 hours post-injection. Protein deficiency and Tween 60 in combination increased the rate of absorption of BHA. Excretion of 3H-BHA and/or its metabolites in urine and feces was complete 6.5 days after administration of 2,500 mg/kg 3H-BHA.

Animals↗
PubMed2001

Abnormal activation of glial cells in the brains of prion protein-deficient mice ectopically expressing prion protein-like protein, PrPLP/Dpl.

BACKGROUND: Some lines of mice homozygous for a disrupted prion protein gene (Prnp), including Ngsk Prnp(0/0) mice, exhibit Purkinje cell degeneration as a consequence of the ectopic overexpression of the downstream gene for prion protein-like protein (PrPLP/Dpl) in the brain, but others, such as Zrch I Prnp(0/0) mice, show neither the neurodegeneration nor the expression of PrPLP/Dpl. In the present study, we found that Ngsk Prnp(0/0), but not Zrch I Prnp(0/0) mice, developed gliosis involving both astrocytes and microglia in the brain. MATERIALS AND METHODS: The brains from wild-type (Prnp(+/+)), Ngsk Prnp(0/0), Zrch I Prnp(0/0), and reconstituted Ngsk Prnp(0/0) mice carrying a mouse PrP transgene, designated Tg(P) Ngsk Prnp(0/0) mice, were subjected into Northern blotting and in situ hybridization using probes of glial fibrillary acidic protein (GFAP) and lysozyme M (LM) specific for astrocytes and microglia, respectively. Immunohistochemistry was also performed on the brain sections using anti-GFAP and anti-F4/80 antibodies. RESULTS: Northern blotting demonstrated upregulated expression of the genes for GFAP and LM in the brains of Ngsk Prnp(0/0), but not in Zrch I Prnp(0/0) mice. A transgene for normal mouse PrP(C) successfully rescued Ngsk Prnp(0/0) mice from the glial activation. In situ hybridization and immunohistochemistry revealed activated astrocytes and microglia mainly in the white matter of both the forebrains and cerebella. In contrast, there was no evidence of neuronal injury except for the Purkinje cell degeneration. Moreover, the glial cell activation was notable well before the onset of the Purkinje cell degeneration. CONCLUSIONS: These findings strongly suggest that ectopic PrPLP/Dpl in the absence of PrP(C) is actively involved in the glial-cell activation in the brain.

Aging↗
PubMed1994

Intraperitoneal administration of nucleoside-nucleotide mixture inhibits endotoxin-induced bacterial translocation in protein-deficient mice.

Nucleosides and nucleotides as a precursor for nucleic acid synthesis may be essential for rapidly growing cells, since intestinal epithelial cells have limited capacity for the de novo purine and pyrimidine synthesis. The present study was undertaken to determine the effect of intraperitoneal administration of nucleoside-nucleotide mixture (NNM) or saline on endotoxin-induced bacterial translocation, ileal histology, and cecal population levels in protein-deficient mice. Intraperitoneal administration of NNM for 14 days was associated with reduced translocation of gram-negative enterics to the mesenteric lymph node and spleen in comparison to saline. Histologically, the extent of the damage to the gut mucosa was greater in the saline group. This was confirmed by the profound diminution of the villous height, crypt depth, and the intestinal wall in the saline treated group as compared to the NNM treated group, suggestive of the efficacy of NNM in improving the gut and epithelial mucosal cells. However, the cecal population levels in both groups were not different. Additionally, the mice in the saline group were more susceptible to the lethal effects of endotoxin as compared to the NNM group suggesting that NNM may be essential for the enhancement of the host defense system. These results suggest that NNM may be used to an advantage to inhibit or reduce the incidence of endotoxin-induced bacterial translocation and improved survival in protein-deficient mice.

Animals↗
PubMed1993

[Familial protein S deficiency].

Protein S deficiency is an autosomal dominant inherited disease. The authors describe a family in which 27 of the 52 members had a deficiency of S protein. Eight family members have suffered from thromboembolic disease.

Adult↗
PubMed2003

Reconstitution of gene expression from a regulatory-protein-deficient hepatitis B virus genome by cell-permeable HBx protein.

Various functions are ascribed to the HBx regulatory protein of the hepatitis B virus (HBV). Due to the low expression level of HBx, it has been difficult to correlate spatial and temporal HBx expression levels with specific functions. Based on a novel cell-permeable peptide, known as the translocation motif (TLM), cell-permeable HBx fusion proteins were generated. The TLM-HBx fusion protein is rapidly internalized from the medium into almost all cells, whereas no significant internalization was seen with wild-type HBx. The major fraction of internalized HBx protein moves from the cytoplasm to the nucleus. The cytosolic fraction, however, activates c-RAF1/extracellular-signal-related kinase 2 signalling and causes activation of activator protein 1 (AP1) and nuclear factor-kappaB. The TLM-HBx protein rescues HBV gene expression from an activator-deficient HBV genome. These results indicate that cell-permeable regulatory proteins provide a novel, efficient tool for a clearly defined, dose-dependent analysis of regulatory protein function, without affecting the integrity of the cell, and can be used for the safe reconstitution of virus production from a regulatory-protein-deficient virus genome.

Cell Line, Tumor↗
PubMed1989

A new case of 'type II' inherited protein S deficiency.

Protein S inherited deficiency is associated with high risk of recurrent venous thrombotic disease (Broekmans et al, 1985a, b). Protein S exists as two forms in plasma, either free and functionally active or complexed with C4b-binding protein (C4b BP) and inactive (Dahlbäck & Stenflo, 1981). We report here the case of a 26-year-old woman and her brother, 28 years old, both suffering from recurrent venous thrombosis since the age of 20, diagnosed as severe protein S deficiency according to the following data: free protein S: 2.5-3% by ELISA, undetectable by electroimmunodiffusion (EID); total protein S: 13-16% by ELISA, 21-18% by EID, C4b BP: normal levels. Crossed immunoelectrophoresis using anti-protein S antibodies revealed only traces of protein S associated with C4b BP and no free protein S. All these assays were performed in the absence of any anticoagulant therapy. Among the investigated relatives, less severe protein S deficiency was observed in three children of the propositus: total protein S levels ranging from 41% to 50% (EID), 40-53% (ELISA); free protein S levels ranging from 16% to 18% (EID), 10-12% (ELISA); normal C4b BP levels. Crossed immunoelectrophoresis revealed traces of free protein S but a significant amount of protein S associated with C4b BP. From these results, we consider, according to Comp's classification (Comp et al, 1986a), that the propositus and her brother are the second case of protein S deficiency type II to be reported in the literature while her children belong to the type I category.

Adult↗
PubMed1998

Protein deficiency in a colony of western lowland gorillas (Gorilla g. gorilla)

A syndrome of alopecia and weight loss in a colony of 10 western lowland gorillas (Gorilla gorilla gorilla) in Gabon during a 3-yr period was apparently due to a dietary protein deficiency, with nine individuals affected to some extent. The most severely afflicted was a 4-yr-old female who eventually died as a result of acute gastroenteritis caused by Shigella flexneri. Clinical signs included chronic alopecia, hair discoloration, failure to thrive, and weight loss, and their severity was directly correlated with the degree of hypoalbuminemia (12 g/L in the most extreme case) and normocytic normochromic anemia. Preliminary clinical tests and autopsy results suggested a dietary protein or amino acid deficiency as the cause of the hypoalbuminemia, and further analyses of serum amino acid and protein levels were consistent with a diagnosis of dietary protein deficiency. Supplementation of the colony diet with a protein preparation for humans produced a rapid amelioration of signs and improvement in body and coat condition, a normalization of serum albumin and total protein levels, and disappearance of the anemia in all affected animals except a 12-yr-old male, who responded well to treatment with anabolic steroids. The natural diet of western lowland gorillas is surprisingly high in protein, and the dietary protein requirement of captive gorillas may be increased as a result of the absence of commensal gastrointestinal ciliates.

Alopecia↗
PubMedDate not supplied

[Beta-glucuronidase activity in the gastric juice and gastric mucosa of rats subjected to protein deficiency].

The experiment was performed in order to evaluate the beta-glucuronidase activity in gastric juice and gastric mucosa of rats submitted to protein-free diet. A group of 36 young adult male wistar rats was fed a protein-free diet ad libitum for five weeks; a second group of 36 wistar rats ingested a purified isocaloric 12,5% casein diet for the same period. The concentration of proteins in plasma, gastric juice and gastric glandular mucosa and the beta-glucuronidase activity in the gastric juice and gastric glandular mucosa were determined. Protein deficient rats had lower plasma protein concentration and also a lower protein concentration in gastric juice and gastric mucosa. In these animals there was no significant change of beta-glucuronidase activity in the gastric juice, but there was a significant increase of the specific enzimatic activity in the gastric mucosa. The results suggest that protein restriction in young adult rats affects the gastric mucosa. The increase of the specific beta-glucuronidase activity might be due to heightened local catabolism or to a comparatively more severe protein depletion.

Animals↗